Abstract Objective To evaluate the performance of risk stratification protocols for febrile neutropenia specific to the pediatric population. Methods Retrospective study of a cohort of pediatric patients undergoing cancer treatment with episodes of neutropenia due to chemotherapy and fever, treated at the emergency department of a tertiary cancer hospital from January 2015 to June 2017. Patients who were bone marrow transplant recipients and patients with neutropenia due to causes other than chemotherapy were excluded. Six protocols were applied to all patients: Rackoff, Alexander, Santolaya, Rondinelli, Ammann 2003, and Ammann 2010. The following outcomes were assessed: microbiological infection, death, ICU admission, and need for more than two antibiotics. The performance of each protocol was analyzed for sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and receiver operator characteristic (ROC) curve. Results This study evaluated 199 episodes of febrile neutropenia in 118 patients. Microbiological infection was identified in 70 samples from 45 distinct episodes (22.6%), 30 patients used more than two antibiotics during treatment (15%), eight required ICU admission (4%), and one patient died (0.8%). Three protocols achieved high sensitivity indices and NPV regarding the outcomes of death and ICU admission: Alexander, Rackoff, and Ammann 2010; however, Rackoff showed higher sensitivity (0.82) and NPV (0.9) in relation to the microbiological infection outcome. Conclusion The Rackoff risk rating showed the best performance in relation to microbiological infection, death, and ICU admission, making it eligible for prospective evaluation.

RESUMO Objetivo Descrever a prevalência dos distúrbios da comunicação, deglutição e motricidade orofacial em um grupo de crianças e adolescentes, no momento da matrícula hospitalar em um instituto oncológico. Método Estudo transversal, com a inclusão de crianças com dois anos ou mais e adolescentes de ambos os gêneros, matriculados na Seção de Oncologia Pediátrica do Instituto Nacional de Câncer José de Alencar Gomes da Silva (INCA) no período de março de 2014 a abril de 2015 para investigação e/ou tratamento de tumores sólidos. Foi utilizado um protocolo para registro das informações sociodemográficas e clínicas e os achados da avaliação clínica fonoaudiológica, que contemplava aspectos do sistema sensório-motor oral, deglutição, fala, linguagem, voz e audição. Resultados Oitenta e oito crianças/adolescentes (41,3%) avaliados apresentavam algum tipo de distúrbio fonoaudiológico. As alterações fonoaudiológicas mais frequentes foram o distúrbio miofuncional orofacial, a disfonia e os transtornos de linguagem. Os menos frequentes foram a disacusia, a paralisia de língua e o trismo. A variável clínica que teve associação estatisticamente significante com a presença de alterações fonoaudiológicas foi o local da lesão. Conclusão O estudo demonstrou uma alta prevalência de alterações fonoaudiológicas em crianças e adolescentes no momento da matrícula hospitalar em um hospital oncológico. A ocorrência de transtornos fonoaudiológicos foi maior nos grupos de participantes com lesões localizadas no sistema nervoso central e na região da cabeça e pescoço.

ABSTRACT Purpose Describe the prevalence of communication, swallowing and orofacial myofunctional disorders in a group of children and adolescents at the time of registration at a cancer hospital. Methods A cross-sectional study conducted with children aged ≥2 and adolescents, of both genders, admitted to the Pediatric Oncology Section of the Instituto Nacional de Câncer José de Alencar Gomes da Silva (INCA) from March 2014 to April 2015 for investigation and/or treatment of solid tumors. A protocol was used to record the sociodemographic and clinical information and findings of the speech-language pathology clinical evaluation, which included aspects of the oral sensorimotor system, swallowing, speech, language, voice, and hearing. Results Eighty-eight children/adolescents (41.3%) presented some type of speech-language disorder. The most frequent speech-language disorders were orofacial myofunctional disorder, dysphonia, and language impairments, whereas the less frequent ones were dysacusis, tongue paralysis, and trismus. Site of the lesion was the clinical variable that presented statistically significant correlation with presence of speech-language disorders. Conclusion High prevalence of speech-language disorders was observed in children and adolescents at the time of admission at a cancer hospital. Occurrence of speech-language disorders was higher in participants with lesions in the central nervous system and in the head and neck region.

Abstract The authors present a proposal of a partnership between the Sociedade Brasileira de Oncologia Pediátrica (SOBOPE) and the International Society of Pediatric Oncology (SIOP) to promote the standardization and improvement of nutritional care of kids under cancer treatment in Brazil. The results of the first meeting in Brazil as well as plans for future meetings are described.

RESUMO O presente trabalho é um relato de caso que descreve os achados fonoaudiológicos e de qualidade de vida apresentados por uma adolescente com um tumor do sistema nervoso central acometida pela mielinólise extrapontina. A mielinólise extrapontina é uma doença desmielinizante aguda que pode ser causada por variações abruptas na osmolaridade sérica, como o que ocorre na rápida correção da hiponatremia. Os dados foram obtidos a partir da avaliação clínica fonoaudiológica, de questionários pediátricos sobre a qualidade de vida e dados contidos no prontuário médico. A paciente apresentou mutismo, disartria e disfagia decorrentes da mielinólise extrapontina, com impactos significativos na sua comunicação verbal, alimentação e qualidade de vida. Os escores de qualidade de vida após a mielinólise extrapontina apresentaram piora quando comparados aos do período anterior à doença. Recebeu atendimento fonoaudiológico hospitalar e ambulatorial. Foram observadas evoluções nos padrões de fala, deglutição e mobilidade das estruturas orofaciais, com reversão parcial dos déficits neurológicos. A atuação fonoaudiológica, como parte de uma equipe multiprofissional de saúde, é de grande importância na reabilitação funcional dos pacientes acometidos por esta doença.

ABSTRACT This paper is a case report describing the speech-language and quality of life outcomes presented by a teenager with a central nervous system germ cell tumor affected by extrapontine myelinolysis. The extrapontine myelinolysis is an acute demyelinating disease that can be caused by abrupt changes in serum osmolality, as in the rapid correction of hyponatremia. Data were obtained from clinical assessment protocols, pediatric inventory about quality of life and medical records information. The patient presented mutism, dysphagia and dysarthria caused by extrapontine myelinolysis, with significant impacts on their verbal communication, feeding and quality of life. The scores of quality of life after extrapontine myelinolysis decreased compared to the time before the disease. She received speech-language therapy during hospital admission and in the outpatient clinic. We observed changes in patterns of speech, swallowing and mobility of orofacial structures; with partially reverse of neurological deficits. The speech therapists, as part of a multidisciplinary team of health care, are of great importance in the functional rehabilitation of patients affected by this disease.

OBJECTIVES: Childhood cancer mortality has substantially declined worldwide as a result of significant advances in global cancer care. Because limited information is available in Brazil, we analyzed trends in childhood cancer mortality in five Brazilian regions over 29 years. METHODS: Data from children 0-14 years old were extracted from the Health Mortality Information System for 1979 through 2008. Age-adjusted mortality rates, crude mortality rates, and age-specific mortality rates by geographic region of Brazil and for the entire country were analyzed for all cancers and leukemia. Mortality trends were evaluated for all childhood cancers and leukemia using joinpoint regression. RESULTS: Mortality declined significantly for the entire period (1979-2008) for children with leukemia. Childhood cancer mortality rates declined in the South and Southeast, remained stable in the Middle West, and increased in the North and Northeast. Although the mortality rates did not unilaterally decrease in all regions, the age-adjusted mortality rates were relatively similar among the five Brazilian regions from 2006-2008. CONCLUSIONS: Childhood cancer mortality declined 1.2 to 1.6% per year in the South and Southeast regions.

The most frequent epigenetic alterations in Wilms tumor (WT) occur at WT2, assigned to 11p15. WT2 consists of two domains: telomeric domain 1 (DMRH19) that contains the IGF2 gene and an imprinted maternally expressed transcript (H19) and centromeric domain 2 (KvDMR) that contains the genes KCNQ1, KCNQ1OT1 and CDKN1C. In this work, we used pyrosequencing and MS-MLPA to compare the methylation patterns of DMRH19/KvDMR in blood and tumor samples from 40 WT patients. Normal constitutional KvDMR methylation indicated that most of the epigenetic alterations in WT occur at DMRH19. Constitutional DMRH19 hypermethylation (HM DMRH19) was observed in two patients with Beckwith-Wiedemann syndrome. Pyrosequencing and MS-MLPA showed HM DMRH19 in 28/34 tumor samples: 16/34 with isolated HM DMRH19 and 12/34 with concomitant HM DMRH19 and KvDMR hypomethylation, indicating paternal uniparental disomy. With the exception of one blood sample, the MS-MLPA and pyrosequencing findings were concordant. Diffuse or focal anaplasia was present in five tumor samples and was associated with isolated somatic HM DMRH19 in four of them. Constitutional 11p15 methylation abnormalities were present in 5% of the samples and somatic abnormalities in the majority of tumors. Combined analysis of DMRH19/KvDMR by pyrosequencing and MS-MLPA is beneficial for characterizing epigenetic anomalies in WT, and MS-MLPA is useful and reliable for estimation of DNA methylation in a clinical setting.

O tumor epitelial de células fusiformes com elemento thymus-like é uma rara neoplasia maligna da tireoide, ocorrendo predominantemente em crianças, adolescentes e adultos jovens. Habitualmente, esse tumor se apresenta como massa tireoideana, sem metástases ao diagnóstico. Acredita-se derivar de arco branquial ou tecido remanescente tímico, apresentando diferenciação tímica primitiva. Este artigo descreve os aspectos clínicos, citológicos, histológicos e imuno-histoquímicos de um caso de SETTLE diagnosticado em uma menina de 3 anos de idade. O aspecto microscópico encontrado no tumor foi de uma lesão nodular, hipercelular, disposta em aspecto bifásico clássico, com componente de células fusiformes, e de tecido glandular acompanhado por epitélio mucinoso e respiratório. O estudo imuno-histoquímico foi positivo para pan-CK, vimentina e actina de músculo liso. Esse caso é o primeiro relato de SETTLE no Brasil. A paciente descrita permanece sem evidência de doença em atividade cinco anos após o tratamento cirúrgico.

Spindle epithelial tumor with thymus-like element (SETTLE) is a rare malignant neoplasm of the thyroid, occurring predominantly in children, adolescents, and young adults. SETTLE usually presents itself as a thyroid mass, without metastases at diagnosis. It is believed to derive from branchial pouch or thymic remnant tissue showing primitive thymic differentiation. This article reports the clinical, cytological, histological and immunohistochemical features of a SETTLE in a 3-year-old girl. Microscopic exam revealed a nodular, highly cellular neoplasm displayed in the classic biphasic pattern, with mixture of prominent spindle cell component and a minor glandular component lined by mucinous or respiratory-type epithelium. The immunohistochemical study showed strong and diffuse positivity for pan-CK, vimentin and smooth muscle actin. The present case is the first SETTLE case reported in Brazil. To date, the patient described remains without evidence of recurrence or metastasis 5 years after surgery.